Primary clear cell carcinoid tumors of the vulva

Neuroendocrine tumors are uncommon in the female genital tract and have been described in the ovary, uterus, cervix, and vagina. Primary carcinoid tumors have not been described in the vulva. We report 3 cases in 3 middle-aged women who presented with a solitary vulvar nodule without any other associated symptoms. All were treated with simple local excision. Two tumors were composed exclusively of clear cells arranged in nests separated by fibrovascular septae. The third tumor predominantly exhibited nests of eosinophilic granular cells with scattered areas of cells showing clear cytoplasm. Immunohistochemical staining for chromogranin and neuron-specific enolase confirmed neuroendocrine differentiation in all cases. Follow-up of 5.5 to 16 years showed no evidence of recurrence or metastasis. Primary clear cell carcinoid tumors of the vulva need to be considered in the differential diagnosis of vulvar masses with clear cell features. Immunohistochemistry plays an important role in the diagnosis of these lesions.